Henoch Schonlein Purpura

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my assignmnet of pediatric in Year 5 IUMP.

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Henoch Schonlein Purpura

  1. 1. Henoch Schonlein Purpura Ahmad Abid Abas 2
  2. 2. What is HSP? <ul><li>Anaphylactoid purpura,Purpura rheumatica, Leukocytoclastic vasculitis. </li></ul><ul><li>It is an inflammatory disorder characterized by a generalized vasculitis involving the small vessels of the skin, GI tract, kidneys, joints, and, rarely, the lungs and CNS. </li></ul><ul><li>Mainly affects children 3-15 yr age.Rarely below 2 yr age. </li></ul>
  3. 3. Etiopathogenesis <ul><li>Unknown etiology. Multifactorial with genetic, environmental and antigenic components. </li></ul><ul><li>More than 75% of patients report antecedent upper-respiratory, pharyngeal, or GI infections. </li></ul><ul><li>An immunoglobulin A (IgA)–mediated autoimmune phenomenon. (clinical manifestation result fr this Ag-Ab complex) </li></ul>
  4. 4. How to Approach ? <ul><li>c/o – Usually the rash is the first to appear and the child is brought to medical attention by his/her parents. </li></ul><ul><li>Hx – Prodromal symptoms such as headache,fever,anorexia. </li></ul><ul><li>O/E – Rash --> Morphology (palpable) ?,(What type cutaneous lesions (petichea,ecchymosis)?,Site of distribution (thighs,buttocks) ? </li></ul>
  5. 5. How to Approach ? <ul><li>Other clinical features to be considered : </li></ul><ul><li>1) Abdomen – crampy abdominal pain. </li></ul><ul><li>2) Joints – migratory arthralgia,arthritis in ankles,knees (big joints) – expect periarticular swelling + significant pain. </li></ul><ul><li>3) Renal – (HTN,edema,oliguria and hematuria +/- azotemia) similar CP of APSGN. </li></ul>
  6. 6. How to Approach ? <ul><li>Dx - Palpable purpura (mandatory) in the presence of at least one of the following four features: </li></ul><ul><li>• Abdominal pain </li></ul><ul><li>• Arthritis (acute) or arthralgia </li></ul><ul><li>• Renal involvement (any haematuria and/or proteinuria) </li></ul><ul><li>• Any biopsy showing predominant Iga deposition </li></ul>
  7. 7. What to be expected in Investigations? <ul><li>Electrolyte values  Normal unless excess vomit. </li></ul><ul><li>BUN/creatinine(RFT) – May ↑ in presence renal involvement. </li></ul><ul><li>CBC – (exclude thrombocytopenia) leukocytosis possibly eosinophilia,Platelet counts N or ↑ .Hb or Ht values may be N or ↓ 2ry to bleeding. </li></ul><ul><li>Urinalysis – ( hematuria, proteinuria, and occasional red cell casts.) (like APSGN) </li></ul>
  8. 8. How to Approach ? Criteria ITP Acute Leukimia Aplastic Anemia HSP Hb/RBC count Normal ↓ ↓ Normal WBC count Normal ↑↑↑ ↓ ↑ in some cases. Platelet count ↓ ↓ ↓ Normal
  9. 9. How to narrow DDx ? <ul><li>Tourniquet test [Hge tendency,capillary fragility] </li></ul><ul><li>Antistreptolysin-O (ASO) titer [recent Strept. infection] </li></ul><ul><li>Monospot test [rapid test for Mono. (EBV)] </li></ul><ul><li>Antinuclear antibody (ANA) test [exclude SLE] </li></ul><ul><li>Rheumatoid factor (RF) test [exclude RA] </li></ul><ul><li>Determination of C3/C4 levels (diff. fr APSGN) </li></ul><ul><li>Prothrombin time (PT) and activated partial thromboplastin time (aPTT) </li></ul><ul><li>Blood cultures [potential infection,meningitis) </li></ul><ul><li>Abd U/S – [Evaluate Intussusception & exclude appendicitis] </li></ul>
  10. 10. How to Manage ? <ul><li>Rx is largely supportive. </li></ul><ul><li>Abdominal pain – Common and self-limited. </li></ul><ul><li>Joint pain – NSAID agents </li></ul><ul><li>Cutaneous manifest. – self-limited may relapse. </li></ul><ul><li>Ch. Cutaneous lesion – colchicine,aspirin,systemic CST. </li></ul><ul><li>No optimal management for GIT and Renal involvement.Should be follow up closely. </li></ul>
  11. 11. Conclusion (clinical point of view) <ul><li>HSP is diagnosed clinically. </li></ul><ul><li>GI involvement is early symptom and renal disease is late symptom. </li></ul><ul><li>If occur in adults , it is more severe. </li></ul><ul><li>Palpable purpura,abd pain and arthritis is clinical characteristic triad of HSP in pediatric. </li></ul>
  12. 12. References <ul><li>1) The Indian Academy of Pediatrics,Bulletin of Rheumatology Chapter IAP September 2010. </li></ul><ul><li>2)http://emedicine.medscape.com/article/804681-clinical </li></ul><ul><li>3) http://www.orpha.net/data/patho/GB/uk-HSP1.pdf </li></ul>

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